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Hypoplastic left heart syndrome

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https://www.readbyqxmd.com/read/29118890/a-novel-technique-for-the-administration-of-sub-ambient-oxygen-in-the-operating-room
#1
Asad A Khawaja, Marco Corridore, Joseph D Tobias
Hypoplastic left heart syndrome (HLHS) is a common form of congenital heart disease with abnormal development of left-sided structures of the heart. As excessive pulmonary blood flow is common prior to palliative surgical procedures, therapeutic maneuvers may be required to decrease pulmonary blood flow and augment systemic cardiac output. Respiratory manipulations to optimize the ratio of pulmonary to systemic blood flow may include techniques to induce hypercarbia or the delivery of sub-ambient concentrations of oxygen (FiO2 less than 0...
October 2017: Cardiology Research
https://www.readbyqxmd.com/read/29096870/hypoplastic-left-heart-syndrome-is-not-a-predictor-of-worse-intermediate-mortality-post-fontan
#2
Billie-Jean Martin, Kandice Mah, Luke Eckersley, Joyce Harder, Charissa Pockett, Daryl Schantz, John Dyck, Mohammed Al Aklabi, Ivan M Rebeyka, David B Ross
BACKGROUND: An increasing proportion of those living with single ventricle physiology have hypoplastic left heart syndrome (HLHS). Our objective was to assess the association between HLHS and outcomes post Fontan operation. METHODS: All pediatric patients who underwent a Fontan procedure at the University of Alberta between 1996 and 2016 were included. Follow-up clinical data collected included early and late surgical or catheter reintervention, echocardiography, and long-term transplant-free survival...
October 30, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29081324/coarctation-of-the-aorta-stenting-via-glidesheath-slender-in-a-newborn-with-recoarctation-early-after-a-norwood-operation
#3
Katarzyna Gendera, Julie Cleuziou, Daniel Tanase
In this article, we report on a newborn with hypoplastic left heart syndrome in whom recoarctation of the aorta was treated with a bare metal stent (Cook Formula 414 Stent) in the early postoperative period after a Norwood procedure. To reduce the risk for scarring and occluding the femoral artery the stent was implanted via 5F Glidesheath Slender sheath.
October 30, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/29063727/nurse-driven-analgesia-and-sedation-in-pediatric-patients-with-univentricular-hearts-requiring-extracorporeal-life-support-after-first-stage-palliation-surgery-a-pilot-study
#4
Jörg Michel, Michael Hofbeck, Ines Gerbig, Vanya Icheva, Ellen Heimberg, Walter Jost, Christian Schlensak, Matthias Kumpf, Gunnar Blumenstock, Felix Neunhoeffer
BACKGROUND: Few data are available regarding requirements of sedation and analgesia in children during extracorporeal life support. AIMS: The aim of this study was to evaluate if children with functionally univentricular hearts on extracorporeal life support after first-stage palliation surgery have higher requirement of analgesics and sedatives compared with children without extracorporeal life support using a goal-directed nurse-driven analgesia and sedation protocol...
December 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29057564/the-effect-of-fetal-hemodynamics-on-fetal-growth-in-single-ventricle-and-transposition-of-the-great-arteries
#5
Tarek Alsaied, Stephanie Tseng, Eileen King, Eunice Hahn, Allison Divanovic, Mounira Habli, James Cnota
INTRODUCTION: As birth weight is a critical predictor of neonatal congenital heart defect (CHD) outcomes, the common problem of poor fetal growth in this population is clinically important but not well understood. The impact of fetal hemodynamics on fetal growth and birth weight in CHD has not been assessed. Combined cardiac output (CCO) has been used to evaluate patients with structurally normal heart but rarely in CHD. Middle cerebral artery and umbilical artery pulsatility indices (PIs) have been used to evaluate the relative distribution of circulation in patients with CHD...
October 23, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/29049702/aortic-root-and-hemiarch-replacement-in-a-patient-with-loeys-dietz-and-hypoplastic-left-heart-syndromes
#6
Eltayeb Mohamed Ahmed, Alexandru Ciprian Visan, Graham Stuart, Serban Stoica
A 15-year-old patient with hypoplastic left heart syndrome underwent 3-stage palliation by the age of 3 years. He was later diagnosed with Loeys-Dietz syndrome. On follow-up imaging, the neoaorta was dilated at 50 mm in diameter. He underwent aortic root replacement with a composite valve conduit and hemiarch replacement, using a boat-shaped Dacron graft. The uncertainty of how univentricular circulation would tolerate long bypass time steered us away from a total arch replacement. His postoperative recovery was uneventful...
October 11, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29035962/effectiveness-of-interstage-home-monitoring-on-healthcare-outcomes-in-infants-with-hypoplastic-left-heart-syndrome-a-systematic-review-protocol
#7
Chelsey Andries, Michelle Palokas
What is the effectiveness of home monitoring on interstage survival rates, weight gain, hospital admissions and cost savings in infants with hypoplastic left heart syndrome?
October 2017: JBI Database of Systematic Reviews and Implementation Reports
https://www.readbyqxmd.com/read/29016838/the-hand1-frameshift-a126fs-mutation-does-not-cause-hypoplastic-left-heart-syndrome-in-mice
#8
Beth A Firulli, Kevin P Toolan, Jade Harkin, Hannah Millar, Santiago Pineda, Anthony B Firulli
Aims: To test if a human Hand1 frame shift mutation identified in human samples is causative of hypoplastic left heart syndrome (HLHS). Methods and results: HLHS is a poorly understood single ventricle congenital heart defect that affects two to three infants in every 10 000 live births. The aetiologies of HLHS are largely unknown. The basic helix-loop-helix transcription factor HAND1 is required for normal heart development. Interrogation of HAND1 sequence from fixed HLHS tissues identified a somatic frame-shift mutation at Alanine 126 (NP_004812...
August 31, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28993061/risk-comparison-for-prenatal-use-of-analgesics-and-selected-birth-defects-national-birth-defects-prevention-study-1997-2011
#9
Julia D Interrante, Elizabeth C Ailes, Jennifer N Lind, Marlene Anderka, Marcia L Feldkamp, Martha M Werler, Lockwood G Taylor, James Trinidad, Suzanne M Gilboa, Cheryl S Broussard
PURPOSE: To compare the use of nonsteroidal anti-inflammatory drugs (NSAIDs) and/or opioids to the use of acetaminophen without NSAIDs or opioids with respect to associations with birth defects. METHODS: We used data from the National Birth Defects Prevention Study (1997-2011). Exposure was self-reported maternal analgesic use from the month before through the third month of pregnancy (periconceptional). Adjusted odds ratios (aORs) were calculated to examine associations with 16 birth defects...
October 2017: Annals of Epidemiology
https://www.readbyqxmd.com/read/28976608/cerebrovascular-response-to-maternal-hyperoxygenation-mh-in-fetuses-with-hypoplastic-left-heart-syndrome-hlhs-depends-upon-gestational-age-ga-and-baseline-cerebrovascular-resistance
#10
Anita Szwast, Mary Putt, J William Gaynor, Daniel Licht, Jack Rychik
OBJECTIVES: Compared to normal fetuses, HLHS fetuses have smaller brain volumes and more brain injury, possibly due to diminished cerebral blood flow and oxygen content. By increasing cerebral oxygen delivery, maternal hyperoxygenation (MH) might improve brain development and reduce brain injury in these fetuses. This study investigates whether gestational age (GA) and baseline cerebrovascular resistance impact response to MH in HLHS fetuses. METHODS: The middle cerebral artery (MCA) pulsatility index (PI), a surrogate measure of cerebrovascular resistance, was assessed in 43 HLHS fetuses...
October 4, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28964428/surgical-interventions-in-infants-born-preterm-with-congenital-heart-defects-an-analysis-of-the-kids-inpatient-database
#11
Jagdish Desai, Sanjeev Aggarwal, Steven Lipshultz, Prashant Agarwal, Paulos Yigazu, Riddhiben Patel, Samantha Seals, Girija Natarajan
OBJECTIVE: To evaluate short-term outcomes in infants born preterm with congenital heart defects (CHDs) and the factors associated with surgery, survival, and length of hospitalization in this population. STUDY DESIGN: We analyzed data from infants born preterm (gestational age <37 weeks) enrolled in the multicenter Kids' Inpatient Database of the Healthcare Cost and Utilization Project who were admitted to the hospital within 30 days after birth. Infants with atrial septal defects were excluded...
September 27, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28957534/combining-computer-modelling-and-cardiac-imaging-to-understand-right-ventricular-pump-function
#12
John Walmsley, Wouter van Everdingen, Maarten J Cramer, Frits W Prinzen, Tammo Delhaas, Joost Lumens
Right ventricular (RV) dysfunction is a strong predictor of outcome in heart failure and is a key determinant of exercise capacity. Despite these crucial findings, the RV remains understudied in the clinical, experimental, and computer modelling literature. This review outlines how recent advances in using computer modelling and cardiac imaging synergistically help to understand RV function in health and disease. We begin by highlighting the complexity of interactions that make modelling the RV both challenging and necessary, and then summarize the multiscale modelling approaches used to date to simulate RV pump function in the context of these interactions...
October 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28938963/study-design-and-rationale-for-elpis-a-phase-i-iib-randomized-pilot-study-of-allogeneic-human-mesenchymal-stem-cell-injection-in-patients-with-hypoplastic-left-heart-syndrome
#13
RANDOMIZED CONTROLLED TRIAL
Sunjay Kaushal, Brody Wehman, Nicholas Pietris, Casey Naughton, Soren M Bentzen, Grace Bigham, Rachana Mishra, Sudhish Sharma, Luca Vricella, Allen D Everett, Kristopher B Deatrick, Sihong Huang, Helina Mehta, William A Ravekes, Naru Hibino, Darcy L Difede, Aisha Khan, Joshua M Hare
Despite advances in surgical technique and postoperative care, long-term survival of children born with hypoplastic left heart syndrome (HLHS) remains limited, with cardiac transplantation as the only alternative for patients with failing single ventricle circulations. Maintenance of systemic right ventricular function is crucial for long-term survival, and interventions that improve ventricular function and avoid or defer transplantation in patients with HLHS are urgently needed. We hypothesize that the young myocardium of the HLHS patient is responsive to the biological cues delivered by bone marrow-derived mesenchymal stem cells (MSCs) to improve and preserve right ventricle function...
October 2017: American Heart Journal
https://www.readbyqxmd.com/read/28936753/long-term-results-of-bilateral-pulmonary-artery-banding-versus-primary-norwood-procedure
#14
Yasutaka Hirata, Hiroaki Miyata, Norimichi Hirahara, Arata Murakami, Hideaki Kado, Kisaburo Sakamoto, Shunji Sano, Shinichi Takamoto
Bilateral pulmonary artery banding (PAB) has emerged to be an attractive option as an initial procedure for the treatment of hypoplastic left heart syndrome (HLHS), and some centers report excellent survival. However, its usage is variable among institutions and its true efficacy is unknown due to reporting biases. We aimed to describe the results of bilateral PAB use, preoperative risk factors, and long-term outcomes compared with primary Norwood procedure, using a national database. Infants who underwent bilateral PAB or Norwood procedure as an initial palliation for HLHS between January 2008 and December 2012 listed in the Japan Congenital Cardiovascular Surgery Database (JCCVSD) were included...
September 21, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28928623/hypoplastic-left-heart-syndrome-with-coronary-cameral-fistulas-echocardiographic-demonstration-of-coronary-artery-steal-and-successful-interventional-treatment
#15
Arshid Mir, Mary Niu, Merick Yamada, Ed Overholt
We report a case of a patient with hypoplastic left heart syndrome with significant coronary-cameral fistulas and exertional symptoms from coronary steal. Symptoms resolved following successful coil occlusion of his left ventricle.
September 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28924546/sirenomelia-associated-with-hypoplastic-left-heart-in-a-newborn
#16
H Turgut, R Ozdemir, I K Gokce, C Karakurt, A Karadag
Sirenomelia, also known as "mermaid malformation/syndrome," is a rare, serious congenital anomaly characterized by variable degrees of fusion of the lower limbs and associated with severe malformations of vertebral, genitourinary, cardiovascular system and single umbilical artery. The first pregnancy of a 25-year-old woman resulted in one twin born by Cesarian section at 32 weeks' gestation, who was referred to our hospital with cyanosis, a congenital anomaly and respiratory distress. On physical examination, there was no urogenital region and anal fissure and gender was indeterminate...
June 30, 2017: Balkan Journal of Medical Genetics: BJMG
https://www.readbyqxmd.com/read/28922269/extubation-failure-is-associated-with-increased-mortality-following-first-stage-single-ventricle-reconstruction-operation
#17
Thomas Scodellaro, Jane M McKenzie, Yves d'Udekem, Warwick Butt, Siva P Namachivayam
OBJECTIVES: To identify the prevalence, causes, risk factors, and outcomes associated with extubation failure following first stage single ventricle reconstruction surgery. DESIGN: Retrospective cohort analysis of neonates who underwent a first stage single ventricle reconstruction operation. Extubation failure was defined as endotracheal reintubation within 48 hours of first extubation attempt. SETTING: The Royal Children's Hospital, Melbourne...
September 15, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28921168/chromosomal-abnormalities-affect-the-surgical-outcome-in-infants-with-hypoplastic-left-heart-syndrome-a-large-cohort-analysis
#18
Dala Zakaria, Xinyu Tang, Rupal Bhakta, Nahed O ElHassan, Parthak Prodhan
Patients with hypoplastic left heart syndrome (HLHS) can have associated genetic abnormalities. This study evaluated the incidence of genetic abnormalities among infants with HLHS and the short-term outcomes of this population during the first hospitalization. This is a retrospective analysis of the multi-center Pediatric Heath Information System database of infants with HLHS who underwent Stage I Norwood, Hybrid, or heart transplant during their first hospitalization from 2004 through 2013. We compared clinical data between infants with and without genetic abnormality, among the three most common chromosomal abnormalities, and between survivors and non-survivors...
September 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28918207/the-association-between-cardiac-physiology-acquired-brain-injury-and-postnatal-brain-growth-in-critical-congenital-heart-disease
#19
Shabnam Peyvandi, Hosung Kim, Joanne Lau, A James Barkovich, Andrew Campbell, Steven Miller, Duan Xu, Patrick McQuillen
OBJECTIVE: To assess the trajectory of perioperative brain growth in relationship to cardiac diagnosis and acquired brain injuries. METHODS: This was a cohort study of term neonates with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA). Subjects underwent magnetic resonance imaging of the brain pre- and postoperatively to determine the severity of brain injury and total and regional brain volumes by the use of automated morphometry...
August 24, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28904928/prenatal-sonographic-diagnosis-of-hypoplastic-left-heart-syndrome
#20
Raghu Teja Sadineni, B Santh Kumar, N B Chander, Durga Mahita Boppana
Hypoplastic left heart syndrome (HLHS) represents a variety of cardiac malformations that may result from errors in the early stages of cardiac development. HLHS includes a wide spectrum of cardiac malformations including hypoplasia of the left ventricle, ascending aorta, hypoplasia, or atresia of the aortic and mitral valves. Over the recent years, the improved resolution of advanced equipment with awareness and increased performance of second-trimester ultrasound examinations for the assessment of fetal anomalies have helped in understanding the spectrum and have expanded our knowledge of HLHS...
July 2017: International Journal of Applied and Basic Medical Research
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